Abstract
It is well established that the endothelin, nitric oxide and prostacyclin pathways play an important role in the development of pulmonary arterial hypertension (PAH). Indeed, the therapeutic options currently available for the management of PAH all act on one of these mechanistic pathways.
However, this is an exciting time for both clinicians and scientists, as increased understanding of the mechanisms involved in the pathogenesis and progression of PAH has resulted in the development of a number of novel therapeutic options.
This article highlights how the introduction of new compounds such as macitentan, riociguat and selexipag, which act on the endothelin, nitric oxide and prostacyclin pathways, respectively, have the potential to further improve the prognosis for patients with PAH.
Footnotes
Provenance
Publication of this peer-reviewed article was supported by Actelion Pharmaceuticals Ltd, Switzerland (principal sponsor, European Respiratory Review issue 126).
Statement of Interest
J-L. Vachiéry has served as a consultant and advisory board member for Actelion, GSK and United Therapeutics. He has received speaker fees from Actelion, Bayer Shering, GSK, Eli Lilly, Pfizer and United Therapeutics. S. Gaine has served as a consultant, advisory board member and has received speaker fees from Actelion, GSK, Pfizer and United Therapeutics.
- Received August 23, 2012.
- Accepted September 21, 2012.
- ©ERS 2012